A 54-year-old female presented to the outpatient Department of Internal Medicine with the history of progressive mild dysphagia for past 3 to 4 months with no history of significant weight loss. She had no significant past history. Her blood pressure, heart rate, respiratory rate, and body temperature were within normal limits. The complete blood counts, hemogram, and coagulation profile were also normal. Her chest radiograph and barium swallow studies were unremarkable.
She was referred for contrast-enhanced computed tomography (CECT) of the neck and chest. The CT was performed using 64-slice Somatom Sensation (Siemens Healthcare, Germany). After the baseline non-contrast study, aortic arch anomalies (aberrant right subclavian artery and truncus bicaroticus) were suspected, and that is why CT aortogram was performed by injecting 90 ml of Iopromide (Ultravist-370) at a rate of 4 ml/s, followed by delayed venous phase to look for the esophageal lumen, neck, mediastinum, and lungs.
The aortic angiography findings were as follows (Figs. 1, 2, and 3):
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(i)
Common origin of right and left common carotid arteries (truncus bicaroticus)
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(ii)
Normal origin and caliber of left subclavian artery
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(iii)
Aberrant origin of right subclavian artery distal and posterior to the origin of left subclavian artery with dilated lumen coursing posteriorly to the right side and compressing upon the mid-esophagus
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(iv)
The vertebral arteries were seen to be arising from the respective subclavian arteries
Fig. 1.
Axial CT angiographic images showing the common origin of bilateral common carotid arteries (arrow, (A)) and posterior origin of right subclavian artery (dot) (A) along with site of compression of mid-esophagus by the dilated posteriorly coursed right subclavian artery (arrow, (B))
Fig. 2.
Coronal reformatted CT angiographic image (A) showing the common origin of bilateral common carotid arteries from arch of aorta (AoA) (truncus bicaroticus). The image (B) shows the corresponding volume rendered technique (VRT) image showing the typical appearance of truncus bicaroticus
Fig. 3.
Coronal Reformatted CT angiographic image (A) showing the origin of right and left subclavian arteries (RSCA and LSCA, respectively). The origin of RSCA is aberrant, posterior, and distal to the origin of LSCA (VRT image (B))
There was no other esophageal lesion or lung lesion seen. There was no evidence of any cervical or mediastinal lymphadenopathy.
With clinical and CT evidence, the diagnosis of dysphagia lusoria due to aberrant right subclavian artery (arteria lusoria) and truncus bicaroticus was formulated. The patient was managed conservatively (keeping in view her mild symptoms and uncomplicated arteria lusoria) by advising softer meals. She was counseled for the need of surgical intervention if the dysphagia worsens.
Arteria lusoria and truncus bicaroticus is a rare combination of aortic arch anomalies. Aberrant right subclavian artery arises due to involution of the right fourth vascular arch with the proximal right dorsal aorta and the persistence of the right seventh inter-segmental artery, which remains attached to the dorsal aorta: after the rotation of the dorsal aorta, the right seventh inter-segmental artery becomes the aberrant right subclavian artery. The artery crosses the midline between the esophagus and vertebral column to reach the right side [1].
It may remain asymptomatic or cause symptoms by compressing the esophagus and trachea resulting in dysphagia or hoarseness of voice. Truncus bicaroticus is considered to be a precursor of symptoms wherein truncus compresses the trachea from front and aberrant right subclavian artery presses the esophagus from behind. Sometimes, the condition may be complicated by the development of an aneurysm in the aberrant artery which may produce additional symptoms due to compression of adjacent organs [2].
The diagnosis can be made by esophagography (demonstration of a pulsatile bulge in the esophageal wall), barium swallow (demonstration of an extrinsic indentation on the posterior esophageal wall), CT angiography, or MR angiography. The CT angiography is the most used modality with high sensitivity for the diagnosis of aberrant right subclavian artery and other aortic arch anomalies. MR angiography is also a well-established modality but in comparison to CT angiography, it is more expensive, takes more time to perform, and requires better patient compliance [3].
The management is conservative in the asymptomatic patients or in patients having mild symptoms. In cases of worsening dysphagia or dyspnea or development of complications like aneurysms, the surgical, endovascular, or combined interventions may be performed [4]. Conventional surgical and endovascular treatments are discussed extensively in the literature but progress of endovascular techniques has led to the hybrid option with combined endovascular and open surgical repair. According to the classification of the lusorian artery pathology, a combined intervention with right subclavian artery transposition, distal or proximal lusorian artery ligation or proximal endovascular occlusion for non-aneurysmal disease, or endovascular thoracic aortic stent graft implantation for lusorian artery aneurysms may be performed [5]. The hybrid method allows exclusion of the artery with thoracic endograft following occlusion of the arteria lusoria via endovascular embolization techniques [6].
Author contributions
It is verified that all the authors had the access to the data and role in the writing of the manuscript.
Compliance with ethical standards
No animals were used in the study. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
Conflict of interest
The authors declare that they have no conflict of interest.
Informed consent
Informed consent was obtained from all individual participants included in the study.
References
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